van der Kooi E L, Kalkman J S, Lindeman E, Hendriks J C M, van Engelen B G M, Bleijenberg G, Padberg G W
Neuromuscular Center Nijmegen, Radboud University Nijmegen Medical Center, 6500 HB Nijmegen, The Netherlands.
J Neurol. 2007 Jul;254(7):931-40. doi: 10.1007/s00415-006-0432-4. Epub 2007 Mar 14.
We recently reported a randomised controlled trial on the efficacy of strength training and the beta2-adrenergic agonist albuterol in patients with facioscapulohumeral muscular dystrophy (FSHD). Strength training and albuterol appeared safe interventions with limited positive effect on muscle strength and volume. We concurrently explored the prevalence and the characteristics of pain and fatigue in the participating FSHD patients, because these are probably underreported but clinically relevant symptoms in this disorder. Next, we studied the effects of albuterol and strength training on pain, experienced fatigue, health-related functional status and psychological distress.
Sixty-five patients were randomised to strength training of elbow flexors and ankle dorsiflexors or non-training. After 26 weeks, albuterol (sustained-release, 8 mg bid) was added in a randomised, double-blind, placebo-controlled design. Outcomes comprised self-reported pain, experienced fatigue, functional status and psychological distress obtained with validated questionnaires at 52 weeks.
Eighty percent of patients reported chronic persistent or periodic, multifocal pains. Thirty-four percent of the participants were severely fatigued. Strength training and albuterol failed to have a significant effect on all outcomes.
Pain and fatigue are important features in FSHD. Strength training and albuterol do not have a positive or negative effect on pain, experienced fatigue, functional status and psychological distress.
我们最近报道了一项关于力量训练和β2-肾上腺素能激动剂沙丁胺醇治疗面肩肱型肌营养不良症(FSHD)患者疗效的随机对照试验。力量训练和沙丁胺醇似乎是安全的干预措施,但对肌肉力量和体积的积极影响有限。我们同时探讨了参与研究的FSHD患者疼痛和疲劳的患病率及特征,因为这些症状在该疾病中可能报告不足但具有临床相关性。接下来,我们研究了沙丁胺醇和力量训练对疼痛、体验到的疲劳、健康相关功能状态和心理困扰的影响。
65名患者被随机分为接受肘屈肌和踝背屈肌力量训练组或非训练组。26周后,采用随机、双盲、安慰剂对照设计添加沙丁胺醇(缓释剂,8毫克,每日两次)。结局指标包括在52周时通过有效问卷获得的自我报告疼痛、体验到的疲劳、功能状态和心理困扰。
80%的患者报告有慢性持续性或周期性、多部位疼痛。34%的参与者严重疲劳。力量训练和沙丁胺醇对所有结局指标均未产生显著影响。
疼痛和疲劳是FSHD的重要特征。力量训练和沙丁胺醇对疼痛、体验到的疲劳、功能状态和心理困扰没有积极或消极影响。